89_1_41

7/27/2019 89_1_41

1/22

Recognition and treatment of Asperger

syndrome in the community

Digby Tantam* and Sobhi Girgis}

Department of Psychiatry, University of Cambridge, Cambridge, UK; Centre for the Study ofConflict and Reconciliation, University of Sheffield, Sheffield, UK, and }Sheffield Health and SocialCare Trust, Sheffield, UK

Sources of data: We conducted a systematic review of the current literature for

this review, but as there are many gaps in the research literature, we have

supplemented this by our own clinical experience.

Areas of agreement: There is a general agreement that Asperger syndrome (AS)

is one of the autistic spectrum disorders, that it is a developmental disorder

which is either present at birth or develops shortly after and that there is a

strong hereditary component.

Areas of controversy: The fundamental impairment of AS is in the social arena,

but what causes this is disputed. We propose that it is a disorder of non-verbal

communication. Another important area of controversy is the extent to which

AS may remit.

Growing points: Many people with AS develop secondary psychiatric disorders in

adolescence and adulthood, some of which may be linked genetically, notably

bipolar disorder [DeLong R, Nohria C (1994) Psychiatric family history and

neurological disease in autistic spectrum disorders. Dev Med Child Neurol, 36,441448] or be explicable by some other association, but many patients and

carers attribute their anxiety and low mood to bullying. The prevalence,

treatment and prevention of co-morbid mental health problems are rapidly

developing areas of interest. Some people with AS are known to commit

offences, and when they commit they are more likely to be violent offences

against strangers. How much of a risk that is presented by people with AS, and

how to assess this risk, is another growing area of concern.

Areas timely for developing research: The social impairments of people with AS

include deficits in empathy, self-awareness and executive function. Many of

these are quintessentially human characteristics, and the study of people with

AS provides opportunities for using neuroimaging to compare people with AS

and controls and identify which areas of the brain are concerned with these

higher functions. The study of AS, like that of other fronto-striatal disorders, is

also throwing light on the role of networks in the brain and on how networks

are formed during embryogenesis.

Keywords: Asperger/diagnosis/detection/community/treatment/management

British Medical Bulletin 2009; 89: 4162

DOI:10.1093/bmb/ldp006

& The Author 2009. Published by Oxford University Press. All rights reserved.

For permissions, please e-mail: [email protected]

Accepted: January 19,

2009

*Correspondence to:

Digby Tantam, Centre for

the Study of Conflict and

Reconciliation, School of

Health and Related

Research, Regents Court,

30 Regent Street,

Sheffield S1 4DA, UK.

E-mail: Djht2@medschl.

cam.ac.uk; D.Tantam@

sheffield.ac.uk

Published Online February 23, 2009

7/27/2019 89_1_41

2/22

Introduction or background

Autism, Asperger syndrome (AS) and the MMR (measles, mumps andrubella) vaccine have become headline news nowadays. The prevalenceof identified autism and identified AS have increased dramatically,

especially the latter, perhaps as much as 11 times.1

Even though therehas probably not been an actual increase in the prevalence of the con-dition, and certainly not of this magnitude2 but an increase in recog-nition only, this greater recognition has led to many more of the publicasking for help and advice about the condition. But what is AS andhow does it fit with other childhood onset problems, such as autisticspectrum disorder (ASD), or pervasive developmental disorder (PDD),all terms that have recently become common in the field? Whathappens to children with autism when they grow up? What role doesthe general practitioner (GP) and the general psychiatrist have in

helping people with autism or one of these related disorders?We answer these and other questions in this review.

What is AS?

AS is named for the Viennese paediatrician Hans Asperger whodescribed autistische Psychopathie in 1944.3 It is now generally con-sidered to be one of the several incompletely differentiated disorders on

the autistic spectrum, a term first introduced by Wing and Gould.4

Asperger himself thought that he was not describing a separate syn-drome from the early childhood autism described by Kanner in 1943,although Kanner disagreed. Although ICD-10 and DSM-IIIR intro-duced separate terms for AS in 1992 and 1987, respectively, repeatedstudies have failed to find criteria validating a differentiation otherthan the arbitrary cutoffs included in the diagnostic criteria. Theseexclusion criteria are: (i) delay in cognitive development (and by impli-cation of mental handicap); (ii) language delay (and by implication ofeither mental handicap or specific language impairment or both);

(iii) onset in the first 3 years of life (DSM-IV only); (iv) absence ofcriteria for making another diagnosis, with a variety of disordersincluding simple schizophrenia being specified.

We advocate the view that there is one, or possibly two, core autisticor pervasive developmental impairments which are present in everyoneon the autistic spectrum, but that many people on the spectrum haveadditional impairments and it is these that distinguish AS from Kannersyndrome.

D. Tantam and S. Girgis

42 British Medical Bulletin 2009;89

7/27/2019 89_1_41

3/22

Core autistic impairment(s)

The triad of social impairments (see Box 1) has been the basis forthe diagnosis of ASDs for over a decade, even though many of itsconstituent elements are hard to define, sometimes inconsistent and

rely on observational judgements that have not been standardized orvalidated. Its values are comprehensiveness and its support of a familyresemblance approach to diagnosis. This allows for the considerablevariation in presenting symptoms of people with AS even when allow-ance is made for the presence of co-morbid impairments.

Box 1 The triad of social impairments.

Consists of impaired

comprehension and use of communication, both verbal and non-verbal;

two-way social interaction; true, flexible, imaginative activities, with the substitution of a narrow

range of repetitive, stereotyped pursuits.

One of us has argued consistently since the mid-1980s5 that the coreautistic impairment is an impairment in non-verbal communication.Other candidates for the core impairment have been put forward in thepastlanguage impairment by Rutter, for example, or anxiety byTinbergenbut none have stood the test of time in the way thatimpaired non-verbal communication has. Recent studies of early diag-

nosis have also supported impaired non-verbal communication as beingone of the best predictors of a later diagnosis of autistic disorder.

Non-verbal communication means the use of gesture, posture, gazedirection, voice prosody, use of space and facial expression to commu-nicate mental contents, such as thoughts and feelings. It overlaps withpragmatics, or the shaping of speech or writing by the needs of theaudience or the desire of the speaker or writer to influence the audiencein a particular direction.

Only a small part of non-verbal communication involves sending orreceiving explicit messages, such as forced, social smiles or convention-

al gestures like waving. Most of it is automatic in that it occurswithout the mediation of planning or self-reflection. Verbal phrases orsentences, in contrast, are formulated from a conscious process offraming a thought, although in practice there is an admixture of non-verbal and verbal communication in any particular utterance orstatement.

Both verbal and non-verbal communications include an element ofencoding and of decoding. People with AS who have a substantial

Recognition and treatment of AS in the community

British Medical Bulletin 2009;89 43

7/27/2019 89_1_41

4/22

difficulty in encoding non-verbal communication, that is in non-verbalexpression, fit best with many peoples mental picture of a person withAS. Their manner may appear odd, because of gait, gestural or posturalidiosyncrasies, their speech may not seem tuned in to the listener andmay, therefore, make them seem self-preoccupied or eccentric, and

their lack of or idiosyncrasies in facial expression may further frustratesocial expectations. Prosodic impairment in speech is often now linkedto speech dyspraxia and it may be that these non-verbal encoding diffi-culties are a reflection of the dyspraxia with which this presentation ofAS may be associated.

Many people with this kind of AS also have decoding impairments.They find it difficult to read faces, for example,6,7 or to infer whatanother person is thinking from the direction of their gaze.8 This maybe due to a very early failure, indeed perhaps the original psychologicalimpairment to orientate the eyes, and to their direction of gaze.9 It isnot clear whether these impairments in non-verbal interpretationamount to a second core impairment, or whether there is a moregeneral impairment of both encoding and decoding, as is the case forverbal communication in Wernickes aphasia and a second, morespecific disorder of decoding, comparable to Brocas aphasia in verbalcommunication.

Our clinical experience is that there are important differencesbetween those whose main impairment is in decoding, that is, animpairment of non-verbal interpretation, and those with a combinedexpressive and interpretative impairment. In the absence of an accepteddiagnostic category, we have termed the former as atypical AS.

An impairment of non-verbal interpretation leads to a lack ofempathy (not to be confused with a lack of sympathy, that is, with alack of compassion-that may or may not be affected). This does notjust prevent a person from taking other people into account on everyoccasion when to do so is expected; it also reduces a persons persua-siveness leading to a loss of social influence, which can increase socialisolation and frustration. Atypical AS is, in our clinical experience,more likely to coexist with attention-deficit disorder (ADD) andattention-deficit hyperactivity disorder (ADHD) or with dysexecutivesyndrome in the absence of other symptoms of ADD, with specific

expressive language impairment, and with Tourette syndrome.Many people with AS will describe themselves as having friends, but

close enquiry often reveals that these are what others would callacquaintanceships or sometimes one-sided friendships. An increasingnumber of people with AS are making friends because more opportu-nities are available. People with atypical AS may make friends easilywith their age group, unlike people with typical AS, but do not keep



7/27/2019 89_1_41

5/22

them, as they are unaware of how to maintain the friendship throughsymmetrical relationship building (Argyle, 1967).

The lack of peer relationships, coupled with the comfort to bereceived from routine and a desire for eradicating uncertainty throughsystematization, can often lead people with AS into espousing unu-sually narrow, private interests (special interests). These are less devel-oped and, therefore, less easily noted in people with autism, oftenbecome dilapidated as the energy goes out of them in older people with

AS and are often concealed in people with atypical AS who are particu-larly committed to trying not to be different from their age group.

We show some of the distinguishing features of these two types ofAS, and of childhood autism, in Table 1.

The commonsense approach to AS

Although there may be little scientific distinction to be made betweenAS and other ASDs, there is an important functional difference, recog-

nized by carers and people with AS, which has necessitated the devel-opment of separate educational, health and social care for people withAS. This difference may become particularly acute in adolescence,which is often more distressing and stormy for people with AS than forpeople with autism. Parents often put this down to their sons ordaughters ability to compare themselves with others, implying thattheir child has sufficient awareness of their own and of other peopleslives to be able to see the ways in which their own could be improved.Research supports the parents perception. People with autism have animpairment of their theory of mind, that is, their ability to account

for the idiosyncratic knowledge, experience and point of view that eachactor has in any social situation. This impairment of theory of mind,which may be due more to the language impairment than to the autis-tic impairment, affects a persons own ability to recognize that theyhave a point of view as much as their ability to recognize otherpeoples. It is closely linked to the increasingly important medico-legalconcept of capacity, which includes the ability to formulate onesown best interests.

Table 1 Differentiation of some of the ASDs.

Impaired non-verbal

expression

Reciprocated

friendships

Unusual interests Self-awareness

Typical autism Y N Y/lack N

AS Y N/few Y Y

Atypical AS N N/brief Y/hidden Y



7/27/2019 89_1_41

6/22

The commonsense use of AS is to apply the term to a person on theautistic spectrum who has the capacity to make decisions for them-selves and who also has the capacity to recognize their difference fromother people and some of the consequences of this, which are regret-tably often adverse and, therefore, emotionally distressing.

Diagnosis

Impaired non-verbal communication may not be easy to spot, and ifrecognized in a global judgement of oddity, it requires conscious effortto categorize it. Transient impairments of non-verbal communicationmay occur in anger and more lasting ones in depression. These are notdue to impaired capacity, but in impaired performance. Impairedcapacity for non-verbal expressiveness, and possibly for non-verbalinterpretative ability, may occur in long-standing schizophrenia, wherethey form a part of the negative syndrome. The one-off assessment ofnon-verbal communication may, therefore, be sensitive to a diagnosis ofan ASD, but is not specific to it. Its absence should, therefore, call a diag-nosis of AS into question and its presence should be supplemented byother diagnostic criteria before making a definite diagnosis (see Box 2).

Box 2 A flowchart for differentiating the PDDs.

Is there an impairment of non-verbal communication? Then

if there is clear history of normal development until the third year oflife, and epilepsy precedes or accompanies cognitive deterioration, con-sider disintegrative disorder;

if there is generalized learning disability or a disorder of language aswell as speech, consider autistic disorder;

if there is a history of cognitive or language delay which has been over-come, make a research diagnosis of autistic disorder, but considertelling the patient and the carer that they have high-functioning autismor AS;

if there is a lack of empathy out of proportion to other impairments,

consider a diagnosis of atypical AS;

if there is a clear developmental history of the triad, consider a diagno-sis of AS, otherwise consider PDD not otherwise specified.

Important supplementary diagnostic criteria are: (i) evidence that thedisorder began early in life (before the age of 3 is specified by ICD-10)and (ii) an impairment of everyday social interaction. Supportive



7/27/2019 89_1_41

7/22

evidence is provided by avoidance of change, cleaving to routines,special interests, a history of incoordination or topographical disorien-tation, motor stereotypes, unusual sensory behaviour (hypersensitivity,unusual fears or fascinations with sound and absorption in one sensoryexperience to the exclusion of all others) and pragmatic errors in

language including concrete use of language, a lack of understandingof jokes that require paralinguistic cues or commonsense to beapplied, inability to understand idioms, inability to change idiolect tosuit social setting (sociolinguistic impairment), problems with pronounsand other shifters and the boiler-plating of catch or imitated phrasesinto conversation without, apparently, fully understanding them.

Prevalence

The inclusion of AS in the PDDs, changing diagnostic criteria andgreater awareness make it difficult to compare the prevalence rates overtime.2 Fombonne has formerly concluded in a review of what is now asubstantial literature that it is unlikely that there has been no realincrease in the condition itself, only its recognition,10 but in his mostrecent summary (ibid.) concludes that Autism has a strong geneticbasis but the possibility of additional causal environmental risk factorsremains . . . If environmental risks contribute to the increase in inci-dence, their impact must occur at or shortly after conception but nosolid clues are yet available. There is a strong public perception thatthere has been a substantial increase, and exposure to the MMR

vaccine has been blamed. Despite repeated investigation having failedto find an association,11 this led to a fall in uptake in routine childhoodvaccination12 and is reflected in a growing demand for specialized edu-cation. Epidemiological studies conducted in different countries haveshown more or less comparable prevalence rates for ASDs. A recentmeta-analysis of 40 studies13 estimated a prevalence rate of 7.1 per10 000 for autistic disorder itself and 12.9 per 10 000 for all otherASDs, including AS. Confidence intervals were wide, and ameta-analysis of US studies with a total base population of over400 000 8 year olds concluded that ASD affects a higher proportion of

8 year olds:14

1 child in every 150. The latter is the rate currently usedby the UKs National Autistic Society. This rate is comparable to theprevalence rates for PDD of 3.16.0 per 1000 children found in tworecent European studies.15,16

Rates of AS are more difficult to come by because there remains con-siderable controversy about the distinction between AS and theso-called high-functioning autistic disorder. In one large Finnishstudy, of 5484 8 year olds, this problem was addressed by using four



7/27/2019 89_1_41

8/22

different diagnostic criteria. There was good agreement between threeof these, including DSM-IV and ICD-10. Averaging the three conver-gent rates provides an estimate that AS affects 1 in every 370 childrenaged 8 years.17

It is usually assumed that the PDDs are life-long disorders and it

would, therefore, follow that the prevalence of PDD in adults is thesame as that in children. This would mean that every GP in the UKwith a list size of 2000 might have 13 patients with a PDD, and everygeneral psychiatrist with a catchment area of 50 000 might have 68patients with PDD and a normal IQ in their patch. However, there issome reason to doubt the presumption that there can be no recovery.Asperger himself reported in a talk to the National Autistic Society ofthe UK in 1970 that many of the children that he had diagnosed had agood prognosis and attributed it to the effects of education. Hisdaughter (M. Asperger, personal communication) has explained to oneof us that her father provided these children with an intensive hospital-based programme of therapy and education (perhaps similar to thatprovided in some adolescent units today), and that it was the combi-nation of psychotherapeutic and educational methods that wasbeneficial.

A recently completed survey of Sheffield adolescents and adults withAS18 found a progressive fall in the estimated prevalence with age inmales with AS, which was too substantial to be likely to be accountedfor by increased mortality or institutionalization in the Asperger groupbut was suggestive of remission in a proportion of children with AS.The fall in prevalence was shown only in males: AS was just as preva-

lent in older women as in teenagers, although at a lower rate than inboys and men.

Autism is more prevalent in males than females with a ratio of 3:1 or4:1. In studies using broader definitions,9 the ratio is reported to varylinearly with IQ reaching up to 10:1 in the more able group. In theSheffield survey (ibid.), the overall ratio of men to women was 4:1. Justunder a half (43.5%) of the surveyed sample had never had a diagno-sis. The male:female ratio in this undiagnosed group was much lower(1.8:1). This suggests that male gender increases the chances of diagno-sis, and that AS in women is underdiagnosed, possibly because of the

hypothesis recently put forward by influential researchers19

that AS isan extreme form of maleness. It also suggests that diagnostic practicecurrently has an inbuilt gender bias.

The failure to diagnose, or to diagnose sufficiently early, remains thegreatest concern of carers because of its ramifications for understand-ing, services and treatment (see Box 3).



7/27/2019 89_1_41

9/22

Box 3 Consequences of failure to diagnose.

In children

Absence of early intervention

Family without an explanation of difficulties

Inappropriate schooling

Unawareness of risk of social, emotional and behavioural problems.

Failure to provide genetic screening

In adults

Family distress/spousal distress

Failure to meet social and emotional needs: work support, socialinclusion, benefits entitlement/use of community resources, support inchild care

Failure to divert affected offenders from imprisonment to a therapeuticdisposal

Co-morbidity

Medical

There is an increased association between having an ASD and a varietyof other medical conditions (see Boxes 4 and 5). The likelihood ofhaving a diagnosable co-morbid physical condition is greatest for autis-tic disorder and least for AS.

Box 4 Medical conditions reported in association with AS.

1. Tuberous sclerosis

2. Neurofibromatosis

3. Hypothyroidism

4. Congenital deafness

5. Congenital visual impairment

6. Epilepsy

7. Neuromuscular disorders8. Connective tissue disorders affecting muscle function

9. Hereditary disorders affecting glucocorticoid levels

10. Fragile X syndrome

11. Sotos syndrome

12. A variety of chromosomal disorders



7/27/2019 89_1_41

10/22

Box 5 Key points.

Autistic disorder, Asperger syndrome (AS) and other pervasive devel-opmental disorders shade into each other.Current evidence is that they are all neurodevelopmental disorders

with a strong hereditary component, but only Rett syndrome has aknown cause.People with AS are prone to emotional problems and this mayreduce their quality of life below that of apparently less able peopleon the autistic spectrum.Early diagnosis and early intervention can produce a reduction inthe impairment caused by the condition and can probably reducethe emotional consequences of the disorder both for the sufferer andfor their families and carers.The GP is still the first professional that many families consult about

diagnosis.In children, developmental delays in socialization, language andmotor coordination and, in adults, a life-long difficulty in makingfriends indicate the need for further assessment for ASD.

Epilepsy is more likely in people with AS than in the general popu-lation,20 although reported rates vary, possibly because the definitionof epilepsy also varies. Fits, when they do occur, are more likely to becomplex than in the total population of people with epilepsy and in atleast some cases may be due to temporal lobe scarring. Temporal

lobectomy rarely reduces the severity of the autistic spectrumsymptoms.21,22

Neuropsychological

There is an increased risk of developmental dyspraxia in people withAS,23 perhaps particularly in the most typical group. Dyspraxia is aclinically mixed syndrome. Some affected individuals may haveimpaired topographical orientation, but other people with AS may beexcellent with maps and in memorizing routes. Fine motor control may

be impaired but not always. The most constant finding is impairmentof whole body coordination, often affecting eye hand coordinationand in younger children, dressing, shoelace tying and other tasksrequiring bilateral coordination.

There is an increased risk of ADD2426 and disorders that are associ-ated with ADD and ADHD, such as dyslexia, specific language impair-ment and Tourette syndrome. Dysexecutive impairment may occur inthe absence of the other elements of ADD.27 This may manifest in



7/27/2019 89_1_41

11/22

reduced working memory, reduced ability to plan and budget, aninability to multi-task because of an inability to keep ones place inan ongoing task that has been put on hold and a reduced ability tocarry sequential tasks. Dysexecutive syndrome may be subtle and easilymissed, but can have disabling effects on everyday living.

Psychiatric

In our own unpublished series of cases seen in our clinic (amounting tosome 1200 people), schizophrenia is no more common than that wouldbe expected, but bipolar disorder is more common as is, probably,obsessive compulsive disorder. Obsessive compulsive disorder withcharacteristic ego alien compulsions can usually, but not always, be dis-tinguished from the rituals and routines that many people with AS con-sidered valuable ways of dealing with uncertainty. Depression may bemore common, although the shifting diagnostic criteria and its fre-quency in the general population make it uncertain, how much morecommon it is. Anxiety and anxiety-related disorders, of clinically sig-nificant severity, have been diagnosed before presentation in over 40%of our clinical attenders. Although the life-time risk of anxiety-relateddisorders is high, most of those attending our clinic are in their teensand early 20s and this rate is likely to be elevated. In our series, thetype of anxiety disorder reflects the age at which the anxiety developsand to some degree, early experience. Dysmorphophobiaa self-critical preoccupation with the size or shape of a bodily partis often

overlooked, but may be an early reaction to the growing perception forsome people with AS of being the odd one out. Anorexia nervosa andagoraphobia may also occur, but social phobia is probably the mostcommon anxiety-related disorder, and may contribute to social with-drawal and a lack of social practice that may otherwise amelioratesocial impairment. Clinical experience suggests that the risk isincreased by bullying and victimization.

In our experience, deliberate self-harm, suicide and substanceabuseall of them particular problems in adolescenceare lesscommon in adolescents with AS. However, they may all occur and

should not be forgotten. Alcohol abuse may be more common in olderadults with AS.

Catatonia occurs in ASD28 rarely but possibly more commonly thanin the general population.29 We have had occasional cases in AS, too.It is sometimes neglected because it is considered as an extreme mani-festation of AS by professionals. Benzodiazepine treatment appears tobe effective,30 but has not been systematically evaluated in people withcatatonia and AS, and needs to be so. The causes of catatonia are



7/27/2019 89_1_41

12/22

unknown but the efficacy of benzodiazepines suggests a link withanxiety. Cycloid psychosis, originally described by Kleist as anxietypsychosis, may also occur more commonly in people with AS, at leastjudging by our own case series. The presentation is of a rapid onset,florid psychosis in which there may be first rank symptoms, but which

usually resolves quickly, sometimes with a low dose of antipsychotics.

Experience of AS

The experience of autism is very variable depending on age, languagedevelopment and intelligence. However, everyone with autism seems tofind making close friends difficult, the social world threatening andchange unpredictable and upsetting. Many people with autism livewith high levels of anxiety, much of the time. People with autistic dis-

orders do not get latched into the normal socializing process that otherinfants do, perhaps because of an inborn failure of social relatednessdue to their impaired non-verbal communication.4 As a result they dis-cover the world afresh and this can lead to striking idiosyncrasy andoriginality as well as unexpected errors in social judgement. Peoplewith autism often seem to live in a world of their own and maydescribe themselves as different, lacking some sense that others have,or as being behind a pane of glass. Contrary to common misperception,many are sociable and want to make friends, failing to do so onlybecause of a lack of intuition about how to behave as a friend. The

internet has changed the lives of many people with autism, since itoffers new possibilities of social interaction, which are not so reliant onnon-verbal communication.

Social and emotional problems

People with autism encounter negative social experiences of isolationand marginalization. Bullying at school is very common, and itsemotional impact may persist into adulthood. These difficulties are

most strongly felt in the high-functioning group. In the UK, membersof this high-functioning group are often said to have AS even if theyhave had cognitive or language delay in the past.

Perhaps as a result of their reflection on the injustice of their socialisolation, people with AS may experience depression or anxiety-relateddisorders, such as anxiety states, social phobia, dysmorphophobia andpanic disorder, and these disorders are often worse in adolescence andyoung adulthood.



7/27/2019 89_1_41

13/22

Management

Diagnosis is the first, and possibly the most important step, in manage-ment. The GP should suspect autism in any child with delays inlanguage, socialization and motor development. The general psychia-

trist should suspect autism in any adolescent or adult who has neverhad close friends. A history should be taken from parents and carers,and the observations of other health professionals should be taken intoaccount. Final diagnosis will usually require a specialist referral.

The fundamental impairment in ASD is a subtle one, possiblybecause non-verbal communication rarely receives direct attention orcomment. Parents or grandparents may often be the first to recognizethat there is something wrong that profoundly affects their contactwith the affected child. Health professionals find such non-specificcomplaints difficult to respond to and are reluctant to diagnose autism

at an early age. More structured observations may help to make a diag-nosis in the less able, more severely affected group. Retrospective ana-lyses of home videos suggest that gaze behaviour is a particularly goodclue,31 and this and other possible signs of autism have been includedin the Checklist for Autism in Toddlers (CHAT) that has been trialledas a screening tool in general practice. The CHAT completed at 18months of age was successful in correctly assigning 65% of autisticchildren,32 but did not reliably predict the later overt development ofthe symptoms of AS, suggesting that this diagnosis cannot yet be madereliable at an early age. A new screening test suitable for AS is currentlyunder development.33

Authoritative guidelines have been published in the last few years cov-ering the screening, assessment and management of autism. The mostrecent in the UK are those produced by the Scottish IntercollegiateGuidelines Network, the Royal College of Psychiatrists and theNational Autistic Society (the National Autism Plan for Children). TheRoyal College of Speech has produced guidelines about the provision ofspeech and language services, which include services for people withASDs. Many of these guidelines, although they mention AS, havelimited application to this condition and to adults in general. Similardocuments have been produced in many other countries, for example

that of the American Academy of Neurology. All of them conclude thatclinical judgement continues to be the only reliable basis for diagnosis,although many suggest supplementary structured interviews that maybe used, and the Scottish guidelines advise that close attention to eitherDSM-IV or ICD-10 criteria is advisable.

The American Academy guidelines notes that parents concernsabout abnormal development are both sensitive and specific, although



7/27/2019 89_1_41

14/22

an absence of concern should not be assumed to mean that the childsdevelopment is normal. They recommend that parents should bespecifically asked about developmental milestones: we would suggestthat particular attention should be paid to social relatedness, languageand speech and motor development. High-risk children, for example

the siblings or children of people with autism, should be particularlycarefully screened. The guidelines recognize that AS and some otherPDDs may be missed at an early stage: we suggest that developmentalscreening should, therefore, be repeated at a later than normal date ifparents have later concerns. Finally, the guidelines recognize that diag-nosis requires the use of the whole clinical picture by an experiencedprofessional.

Once diagnosis has been made, other co-morbid conditions also needto be screened for. The yield of intensive investigation is probably toolow for invasive or time-consuming investigations to be carried out inthe absence of a history of symptoms suggestive of the associated dis-order being considered. However, a careful and systematic historyshould be taken from both the person with AS and an informant.Observation of the patient can occur alongside history-taking, and theobserver should pay attention to any overt neurological signs, to skinlesions that might suggest tuberous sclerosis, to evidence of hearing orvisual impairment, both of which may be associated with AS. CAT orMRI is not routinely justified, although should be considered if thereare any neurological signs. An EEG will often show non-specificabnormalities but these are unlikely to be of clinical significance in theabsence of seizures.

Psychometry, particularly IQ testing and testing frontal lobe function,can be useful in planning education and sometimes in advising aboutemployment.

Intensive social training programmes in early childhood have hadenthusiastic endorsements, although full independent evaluationremains to be carried out. The results suggest that if communicationwith an autistic child can be established, the childs social developmentcan be accelerated and sometimes brought back to its normal trajec-tory, despite the persistence of the underlying neurobiologicalabnormality.

Whether or not a child attends special school remains contentious,but what is clear is that a child with autism has special educationalneeds. These include having teachers who know how to communicatewith an autistic person, attention to social and emotional understand-ing in the curriculum and an active programme of integration withother children, with close attention to detecting and combating bully-ing. People affected by autism and their families should be told theresults of their assessment in language that they can understand, and



7/27/2019 89_1_41

15/22

this should be followed up by a letter that can be used in future consul-tations. Counselling should be available for people with autism or theirfamily members who have developed emotional problems as a result ofthe condition.

People diagnosed for the first time in adolescence or adulthood, and

their carers, also need access to education about the condition. Peoplewith autism are often competent to make decisions and they should beconsulted about the extent to which their carers will be involved intheir health care.

Mental health problems are more common in people with autism.The emergence of psychotic symptoms in an individual with autismdoes not necessarily indicates the onset of a long-term psychotic illness,as brief psychotic episodes are not uncommon. Schizophrenia is rare inpeople with autism, and the most common recurrent psychosis isbipolar disorder, which also appears to be overrepresented in thefamilies of people with PDD, especially AS. The most common mentalhealth problem is depression or an anxiety-related disorder. Peoplewith AS are unusually sensitive to change and they may experience lossparticularly acutely. It is worth considering an emotional problemwhenever there is an otherwise unexplained deterioration in the beha-viour or social functioning of a person with AS.

It is not uncommon that adults with undiagnosed AS present for thefirst time with domestic or occupational difficulties. Their social andemotional disability might cause considerable strain in their relation-ship with their partners at home or their colleagues at work. Asparents, they might have difficulty in picking up cues of their children

being upset, frightened or in pain. Their limited sociability is likely tolimit the amount of social interaction their children would experienceunless this is compensated for by other adult carers.

Medication

There is a nave, but persistent, view that many people with ASDs canbe helped by medication, and almost all of the classes of psychotropicdrugs have been advocated with antipsychotics being particularly often

recommended. Clinical trials sometimes seem to support the value ofmedication, but only if the placebo effect of trial involvement is nottaken into account. We cannot recommend the routine use of anymedication to treat AS. People with AS can sometimes experience asudden rise in arousal that has similarities to the catastrophic reactionsseen in dementia or after a cerebro-vascular episode.34 This can resultin aggression, self-harm or other actions that put the person concernedor those around them at risk. Underlying depression may be a cause, in



7/27/2019 89_1_41

16/22

which case it can be treated with an antidepressant or with cognitivetherapy, but sometimes the cause simply seems to be an inexorable risein subjective anxiety with an increasing desire for release. Sedative anti-psychotic drugs and anticonvulsants have both been used to treat thiscondition, and some clinicians have found them to be effective in pro-

ducing sedation and reducing the frequency of reactions. In the absenceof evidence either for their effectiveness compared with psychologicalcare or for how long they remain effective, they should be consideredas a last resort, and their continuation should be regularly monitored.

The development of catastrophic reactions, an increase in stereotypeor an increased difficulty with change or the re-appearance of beha-viours from an earlier period of development should initiate anassessment for a co-morbid disorder, with particular emphasis onanxiety-related disorder. The usual symptoms and signs of anxiety ordepression, such as poor concentration, new onset sleep disturbance,suicidal thoughts, onset or worsening of self-harm or complaints offeeling tense or low should, of course, also be taken seriously and fol-lowed through. If a diagnosis has been made, medication or psycho-logical treatment should be recommended according to currentlyavailable guidelines for the co-morbid condition. In our experience,selective serotonin reuptake inhibitors are usually well-tolerated andeffective treatments for anxiety and anxiety-related disorder when com-bined with psychosocial intervention. Antipsychotics are effective forthe treatment of psychosis, but may not need to be maintained beyondthe remission of symptoms if the sudden onset and high levels ofanxiety suggest that the psychosis is of cycloid type. Many people with

AS have some degree of phobia about medical intervention, andlithium levels may prove difficult to monitor. An atypical antipsychotic(in children) or semisodium valproate may be, therefore, preferable asa maintenance medication, although there is an absence of research inthis area, and current guidelines should be consulted.

Empathy training, social skills training and desensitization to hyper-aesthesiae may all be effective in studies, but have yet to find a place inroutine clinical practice. One reason may be that interventions even ifeffective in one setting may not generalize to others. An alternativestrategy is to provide counselling or psychotherapy, which draws on

behavioural, cognitive and re-training principles but does so in thecontext of solving real problems in everyday life.

Mental health services

The high prevalence of co-morbid disorders means that there will be aneed for psychiatric input (for both the service and the individual)



7/27/2019 89_1_41

17/22

together with a framework that ensures their close coordination. Theservices for people with AS remain patchy. There is lack of clarity as towhere the disorder fits within the current structure of mental healthservices. Adults with the disorder could be deemed as not eligible forservices from either adult mental health or, by virtue of their IQ, the

learning disabilities services. The Royal College of Psychiatrists (2006)concluded that all psychiatrists should be able to recognize the syn-drome, diagnosing it in clear-cut cases, and be aware of its impli-cations. The College recommended postgraduate training at all levelsfrom basic psychiatric training through to continuing professionaldevelopment. For those cases where diagnosis is less straightforward,or where clinical management is more complex, there needs to beaccess to local specialist expertise and, where necessary, to tertiaryspecialist services. A governmental report (2006) suggested that peoplewith ASDs who have received a service from CAMHS (Child andAdolescent Mental Health Centre) and do not fit the criteria forongoing care in adult mental health should be discharged back to theirGP when they reach 18 years. In a subsequent document (Departmentof Health, 2006), the government made it clear that the current pos-ition, whereby some people with an ASD fall through local services,in particular between mental health and learning disability services, isunacceptable and contrary to the intention of government policy. Thereport emphasized the importance of proper assessment, identifyingneeds and meeting those needs through the most competent approachrather than deciding on which service should provide all those needs,individualized care and social inclusion. The report encourages the pro-

vision of the new approaches to funding and support, such as directpayments and individual budgets to people with ASD in the same wayas everyone else.

Mental capacity and consent

The Mental Capacity Act (2005) stipulates that a person is assumed tohave capacity unless it is established that he/she lacks capacity. Aperson is not to be treated as unable to make a decision unless all prac-

ticable steps to help him/her do so have been taken without success.The assessment of mental capacity of people with AS and the validity of

their consent to treatment may be complicated by a number of features ofthe disorder. These include difficulties with comprehension which may bemasked by an apparent good use of language, difficulties with attentionand concentration, problems in coping with change leading to extremeaversion to anything new and an impaired theory of mind resulting in arigid unrealistic perception of how the world works. As we have already



7/27/2019 89_1_41

18/22

said, people with AS normally have capacity. The most exception is, inour experience, when fear of change prevents a person with AS fromaccepting something new, even if that is in their best interests.

People with AS coming in contact with the criminaljustice system

While many people with the disorder are reliable witnesses, AS canaffect the ability of someone to give an accurate account of an eventbecause of such factors as idiosyncratic misinterpretations of events,difficulties with time dimension, difficulties in distinguishing ownactions from others, inability to cope with formal interviews becauseof unfamiliarity, problems with understanding others and expressingthemselves both verbally and non-verbally and consequently giving

wrong statements or wrong impressions, undue compliance and a ten-dency to stick rigidly to their own accounts even if they were inaccur-ate. They might not be recognized as vulnerable adults because of theiracademic abilities. People with AS are by definition suffering from amental disorder. Under the Police and Criminal Evidence Act 1984,they are entitled to have an appropriate adult present when they areinterviewed by the police.

Some people with AS would, sadly, find the structured environmentappealing. However, many are vulnerable people who find themselvesin conflict with the structure and at risk of serious harm from others

(Scottish Executive, 2004)

Offending

In the Sheffield survey reported previously 81% of respondents said thatthey were easily angered, and 34% reported that they had been violent. Itis a common reason for carers to seek professional help. This is not to saythat every person with AS is violent, but that violence when it occurs canbe particularly problematic, partly because it may not be restrained by

empathic responses to others fear or pain, and partly because it mayappear to be unheralded. The triggers, the warning signs and the contextof different kinds of violence are familiar to most people, but this knowl-edge may not apply to people with AS whose violence may be directedagainst a vulnerable other person and not the one who triggered it, and itmay be temporally remote from its trigger, too.

Some predictors of violence in young people without autism do applyto people with AS, too. A preoccupation with weapons, with violent



7/27/2019 89_1_41

19/22

videos or violent role models, a lack of social influence in everyday lifeand rumination and sometimes rehearsing a violent act may all be pre-dictors, although their non-specificity reduces their value in practice.More work needs to be done in predicting those individuals with ASwho go on to perpetrate very serious violence, including unlawful

killing. Our own anecdotal experience suggests that there is particulardanger when a person feels (i) that neurotypicals are all of a piece,and that their uncaring treatment of the person with AS justifies reta-liation and (ii) that there is nothing to lose. For some people with AS,life imprisonment provides no worse quality of life than the life theyhave in freedom.

Other forensic problems include stalking, and fetishistically relatedoffending, of which paedophilia often presents the greatest concern. Solittle research has been on these problems that no general advice ispossible except that the outrage that the behaviour causes is often oneof the factors that maintains it because it confers a power to have animpact on the person with AS that they find rewarding, howeverharshly they are treated as a result.

What contribution do people with AS make to society?

Most people with AS are still undiagnosed, and it is likely that many ofthem have escaped professional attention because they are living andworking with others. Not everyone with AS needs, or wants, a diagno-sis. Some feel that society should make more efforts to include them,

but others have found ways to integrate into society.We might draw two inferences from this. First, services for people

with AS need to take their contribution to society into account. Somepeople with the condition will need life-long social support, but othersmay need help only during crises, and yet others may never need aspecific intervention at all. It is likely that most of this latter group willbe people who have abilities of value to others that outweigh theirsocial impairments. They may be adults who remain devoted to elderlyparents, while other, non-autistic children will have grown away. Theymay be people with special talents. People with AS have made original

contributions in many fields including the graphic arts and music.Baron-Cohen et al.8 has suggested that academic engineers and mathe-maticians include a higher than expected proportion of people with AS.People with AS may have a special interest in these subjects, but theymay also have an originality, a single-mindedness and an attention todetail that makes them good academics. Not just good academics, butsometimes geniuses, or so it has been suggested: Wittgenstein, Newtonand Einstein have all been retrospectively diagnosed as having AS.



7/27/2019 89_1_41

20/22

Further information about autism

There are many excellent books about autism, AS and other PDDs, manynow written from the different perspectives of the health professional,teacher, employer, spouse, parent, sibling and, increasingly, sufferer.

Many of these titles are published by Jessica Kingsley or by the NationalAutistic Society. Their lists should be consulted for further information.

There are many support groups for parents around the country, and anincreasing number of social groups for people with AS. Details can befound from the National Autistic Society. There is a newsletter publishedby people with AS. Supported employment schemes (ProspectsEmployment Consultancy) specifically for people with AS have been setup in London and the South-East, Sheffield, Glasgow and Manchester.

There are many specialist schools, some now specializing in AS.There is also an increasing amount of specialist residential provision.

Again, the National Autistic Society is a good starting point.There is a considerable amount of information available on the net.There are discussion groups for people with AS, for students with ASand for counsellors of people with AS. Many people with AS have theirown webpages. There is a regular electronic conference about aspectsof autism: . The National Autistic Society site, the Autistic Society ofAmerica (www.autism-society.org) and the OASIS site (www.udel.edu/bkirby/asperger) are all good jumping off places. The Surrey AutisticSociety site contains useful practical information.

Useful medical updates can be found from the CDC webpages.

Acknowledgements

We are grateful to our patients and their carers who have taught us somuch about autism and AS, and about being human. We also thankEmmy van Deurzen for wise counsel and David Taylor for his valuablesuggestions on a draft.

References

1 Wazana A, Bresnahan M, Kline J (2007) The autism epidemic: fact or artifact? J Am Acad

Child Adolesc Psychiatry, 46, 721730.2 Fombonne E (2008) Is autism getting commoner? Br J Psychiatry, 193, 59.3 Asperger H (1944) Die Autistichen Psychopathen in Kindersalter. Arch Psychiatr

Nervenkrankenheiten, 117, 76136.4 Wing L, Gould J (1979) Severe impairments of social interaction and associated abnormalities

in children: epidemiology and classification. J Autism Dev Disord, 9, 1129.



7/27/2019 89_1_41

21/22

5 Tantam D (1986) Eccentricity and Autism: a Clinical and Experimental Study of 60 AdultPsychiatric Patients, Including 46 Able Autistic Adults, with a Social Disability Attributableto Personality or Developmental Disorder. University of London.

6 Hadjikhani N, Joseph RM, Snyder J, Tager-Flusberg H (2007) Abnormal activation of the

social brain during face perception in autism. Hum Brain Mapp, 28, 441449.7 Tantam D, Monaghan L, Nicholson H, Stirling J (1989) Autistic childrens ability to interpret

faces: a research note. J Child Psychol Psychiatry, 30, 623630.

8 Baron-Cohen S, Wheelwright S, Hill J, Raste Y, Plumb I (2001) The reading the mind in theeyes test revised version: a study with normal adults, and adults with Asperger syndrome or

high-functioning autism. J Child Psychol Psychiatry Allied Discipl, 42, 241251.9 Tantam D (1992) Characterizing the fundamental social handicap in autism. Acta

Paedopsychiatr, 55, 83 91.10 Fombonne E (2005) The changing epidemiology of autism. J App Res Intellect Disabil 18,

281294.

11 Lingam R, Simmons A, Andrews N, Miller E, Stowe J, Taylor B (2003) Prevalence of autism

and parentally reported triggers in a north east London population. Arch Dis Child, 88,

666670.

12 Burgess DC, Burgess MA, Leask J (2006) The MMR vaccination and autism controversy in

United Kingdom 1998-2005: inevitable community outrage or a failure of risk communi-

cation? Vaccine, 24, 3921 3928.

13 Williams JG, Higgins JP, Brayne CE (2006) Systematic review of prevalence studies of autismspectrum disorders. Arch Dis Child, 91, 8 15.

14 Anonymous (2007) Prevalence of autism spectrum disordersautism and developmental dis-

abilities monitoring network, 14 sites, United States, 2002. MMWR Surveill Summ, 56, 1228.

15 Baird G, Charman T, Baron C et al. (2000) A screening instrument for autism at 18 monthsof age: a 6-year follow-up study. J Am Acad Child Adolesc Psychiatry, 39, 694702.

16 Ehlers S, Gillberg C (1993) The epidemiology of Asperger syndrome. A total population

study. J Child Psychol Psychiatry, 34, 1327 1350.17 Mattila ML, Kielinen M, Jussila K et al. (2007) An epidemiological and diagnostic study of

Asperger syndrome according to four sets of diagnostic criteria. J Am Acad Child AdolescPsychiatry, 46, 636646.

18 Balfe M, Chen T, Tantam D (2005) Sheffield Survey of Health and Social Care Needs ofAdolescents and Adults with Asperger Syndrome. 13. 2005. Sheffield, School of Health andRelated Research. ScHARR report. Ref Type: Serial (Book, Monograph).

19 Baron CS (2002) The extreme male brain theory of autism. Trend Cogn Sci, 6, 248255.20 Amiet C, Gourfinkel-An I, Bouzamondo A et al. (2008) Epilepsy in autism is associated with

intellectual disability and gender: evidence from a meta-analysis. Biol Psychiatry, 64,547640.

21 McLellan A, Davies S, Heyman I et al. (2005) Psychopathology in children with epilepsy

before and after temporal lobe resection. Dev Med Child Neurol, 47, 666672.22 Tuchman R (2000) Treatment of seizure disorders and EEG abnormalities in children with

autism spectrum disorders. J Autism Dev Disord, 30, 485489.23 Dziuk MA, Larson JCG, Apostu A, Mahone EM, Denckla MB, Mostofsky SH (2007)

Dyspraxia in autism: association with motor, social, and communicative deficits. Dev MedChild Neurol, 49, 734739.

24 Ronald A, Simonoff E, Kuntsi J, Asherson P, Plomin R (2008) Evidence for overlapping

genetic influences on autistic and ADHD behaviours in a community twin sample. J Child

Psychol Psychiatry, 49, 535542.25 Thede LL, Coolidge FL (2007) Psychological and neurobehavioral comparisons of children

with Aspergers disorder versus high-functioning autism. J Autism Dev Disord, 37, 847854.26 Lee DO, Ousley OY (2006) Attention-deficit hyperactivity disorder symptoms in a clinic

sample of children and adolescents with pervasive developmental disorders. J Child Adolesc

Psychopharmacol, 16, 737746.27 Sinzig J, Morsch D, Bruning N, Schmidt MH, Lehmkuhl G (2008) Inhibition, flexibility,

working memory and planning in autism spectrum disorders with and without comorbid

ADHD-symptoms. Child Adolesc Psychiatry Ment Health, 2, 4.



7/27/2019 89_1_41

22/22

28 Kakooza-Mwesige A, Wachtel LE, Dhossche DM (2008) Catatonia in autism: implications

across the life span. Eur Child Adolesc Psychiatry, 17, 327335.29 Wing L, Shah A (2000) Catatonia in autistic spectrum disorders. Br J Psychiatry, 176,

357362.

30 de Winter CF, van DF, Verhoeven WM, Dhossche DM, Stolker JJ (2007) Autism and catato-

nia: successful treatment using lorazepam. A case study. Tijdschr Psychiatry, 49, 257261.

31 Mars AE, Mauk JE, Dowrick PW (1998) Symptoms of pervasive developmental disorders as

observed in prediagnostic home videos of infants and toddlers. J Pediatr, 132, 500504.32 Scambler D, Rogers SJ, Wehner EA (2001) Can the checklist for autism in toddlers differen-

tiate young children with autism from those with developmental delays? J Am Acad ChildAdolesc Psychiatry, 40, 1457 1463.

33 Williams J, Scott F, Stott C et al. (2005) The CAST (childhood Asperger syndrome test): testaccuracy. Autism, 9, 4568.

34 Carota A, Rossetti AO, Karapanayiotides T, Bogousslavsky J (2001) Catastrophic reaction in

acute stroke: a reflex behavior in aphasic patients. Neurology, 57, 1902 1905.35 Argyle M (1967) The psychology of interpersonal behaviour. Harmondsworth, Pelican.

36 Department of Health (2006) Better services for people with an autistic spectrum disorder.

37 Scottish Executive (2004) Antisocial Behaviour etc [Scotland] Act 2004.


62

89_1_41

Documents